Objective To explore the therapeutic effect of different treatment regimens on bleeding and joint mobility in children with hemophilia A(HA).Methods A total of 56 children with moderate to severe HA were divided into on-demand treatment group(n=6),on-demand to prophylaxis group(from on-demand conversion to prophylaxis,n=42)and prophylaxis group(n=8)according to different treatment regimens.They were treated and followed up for 2 years.Each group was collected for general clinical data of HA patients before follow-up[age,classification of hemophilia,time from diagnosis to treatment initiation,treatment duration,FⅧ inhibitors,body mass index(BMI),and treatment regimen switching].Annual bleeding rate(ABR)and annual joint bleeding rate(AJBR)of the patients were recorded.Haemophilic Early Arthropathy Detection with UltraSound in China(HEAD-US-C),Hemophilia Joint Health Score(HJHS),and Hemophilia Functional Independence Score for Hemophilia(FISH)were used to evaluate the joint structure,joint function and joint mobility of the patients,respectively,and the therapeutic efficacy of the patients was simultaneously evaluated.Spearman correlation was used to analyze the association of FISH score with general clinical characteristics,HEAD-US-C and HJHS in the children with HA.Results There were a statistically significant differences in ABR and AJBR scores in on-demand to prophylaxis group between at the beginning and end of the follow-up(P＜0.05).There were statistically significant differences(P＜0.05)in ABR and HJHS scores between patients in prophylaxis group.There were no statistically significant differences in other scores within intragroup(P＞0.05).There was a statistically significant difference in HA patients'age among disease improvement,maintenance and progression groups under HEAD-US-C scoring criteria(P＜0.05).FISH score was negatively correlated with HJHS score(P＜0.05).Further analysis showed that each dimension of FISH score and self-care ability were negatively associated with HJHS score(P＜0.05).Walking ability was negatively correlated with HEAD-US-C and HJHS scores(P＜0.05).Joint function status was a factor affecting the mobility of HA patients.Conclusion The best treatment improvement for ABR and AJBR in HA patients was achieved by switching from on-demand to prophylaxis,followed by prophylaxis.The worst was achieved by on-demand treatment.Joint structure and functional status can affect the joint mobility of HA patients.
关键词
血友病A/关节/儿童/影响因素分析/关节结构及功能/活动功能
Key words
hemophilia A/joint/child/analysis of influencing factors/joint structure and function/motor function
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